Effective treatment with cyclosporine A of a child with systemic lupus erythematosus resistant to cyclophosphamide pulse therapy

Intermittent intravenous cyclophosphamide pulse therapy (IVCY) has been reported to be effective for the treatment of refractory systemic lupus erythematosus (SLE). However, there is a proportion of patients with SLE, who are IVCY-resistant and need a long-term therapy to sustain the remission. We report here a case of a 6-year-old Japanese girl with SLE refractory to IVCY. She suffered from persistent hypocomplementemia and recurrent flares despite receiving methylprednisolone pulse, mizoribine pulse and IVCY therapy. Administration of cyclosporine A (CsA) was, therefore, initiated. Within 2 months of the start of CsA administration, the serum levels of C3, C4 and complement hemolytic activity began to increase rapidly, and finally returned to the normal levels. The serum anti-dsDNA antibody titer was decreased significantly after the initiation of this treatment. The prednisolone dose could be successfully tapered without precipitation of any flares. No adverse effects of CsA were observed. Based on these clinical observations, we suggest that CsA might be an effective treatment option for selected cases of refractory SLE.